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Craniopharyngioma

 

A craniopharyngioma is a noncancerous (benign) tumor that develops at the base of the brain near the pituitary gland.

Causes

The exact cause of the tumor is unknown.

This tumor most commonly affects children between 5 to 10 years of age. Adults can sometimes be affected. Boys and girls are equally likely to develop this tumor.

Symptoms

 

Craniopharyngioma causes symptoms by:

  • Increasing pressure on the brain, usually from hydrocephalus
  • Disrupting hormone production by the pituitary gland
  • Pressure or damage to the optic nerve

Increased pressure on the brain can causes:

  • Headache
  • Nausea
  • Vomiting (especially in the morning)

Damage to the pituitary gland causes hormone imbalances that can lead to excessive thirst and urination, and slow growth.

When the optic nerve is damaged by the tumor, vision problems develop. These defects are often permanent. They may get worse after surgery to remove the tumor.

Behavioral and learning problems may be present.

 

Exams and Tests

 

Your health care provider will perform a physical exam. Tests will be done to check for a tumor. These may include:

  • Blood tests to measure hormone levels
  • CT scan or MRI scan of the brain
  • Examination of the nervous system

 

Treatment

 

The goal of the treatment is to relieve symptoms. Usually, surgery has been the main treatment for craniopharyngioma. However, radiation treatment instead of surgery or along with a smaller surgery may be the best choice for some people.

In tumors that cannot be removed completely with surgery alone, radiation therapy is used. If the tumor has a classic appearance on CT scan, a biopsy may not be needed if treatment with radiation alone is planned.

Stereotactic radiosurgery is performed at some medical centers.

This tumor is best treated at a center with experience in treating craniopharyngiomas.

 

Outlook (Prognosis)

 

In general, the outlook is good. There is an 80% to 90% chance of a cure if the tumor can be completely removed with surgery or treated with high doses of radiation. If the tumor returns, it will most often come back within the first 2 years after surgery.

Outlook depends on several factors, including:

  • Whether the tumor can be completely removed
  • Which nervous system problems and hormonal imbalances the tumor and treatment cause

Most of the problems with hormones and vision do not improve with treatment. Sometimes, the treatment may even make them worse.

 

Possible Complications

 

There may be long-term hormone, vision, and nervous system problems after craniopharyngioma is treated.

When the tumor is not completely removed, the condition may return.

 

When to Contact a Medical Professional

 

Call your provider for the following symptoms:

  • Headache, nausea, vomiting, or balance problems (signs of increased pressure on the brain)
  • Increased thirst and urination
  • Poor growth in a child
  • Vision changes

 

 

References

Alter JL, Kuttesch JF. Brain tumors in childhood. In: Kliegman RM, Stanton BF, St. Geme JW, Schor NF, eds. Nelson Textbook of Pediatrics . 20th ed. Philadelphia, PA: Elsevier; 2016:chap 497.

Styne DM, Grumbach MM. Physiology and disorders of puberty. In: Melmed S, Polonsky KS, Larsen RP, Kronenberg HM, eds. Williams Textbook of Endocrinology . 13th ed. Philadelphia, PA: Elsevier; 2016:chap 25.

Suh JH, Chao ST, Murphy ES, Weil RJ. Pitutary tumors and craniopharyngioma. In: Gunderson LL, Tepper JE, eds. Clinical Radiation Oncology . 4th ed. Philadelphia, PA: Elsevier; 2016:chap 29.

 
  • Endocrine glands - illustration

    Endocrine glands release hormones (chemical messengers) into the bloodstream to be transported to various organs and tissues throughout the body. For instance, the pancreas secretes insulin, which allows the body to regulate levels of sugar in the blood. The thyroid gets instructions from the pituitary to secrete hormones which determine the pace of chemical activity in the body (the more hormone in the bloodstream, the faster the chemical activity; the less hormone, the slower the activity).

    Endocrine glands

    illustration

    • Endocrine glands - illustration

      Endocrine glands release hormones (chemical messengers) into the bloodstream to be transported to various organs and tissues throughout the body. For instance, the pancreas secretes insulin, which allows the body to regulate levels of sugar in the blood. The thyroid gets instructions from the pituitary to secrete hormones which determine the pace of chemical activity in the body (the more hormone in the bloodstream, the faster the chemical activity; the less hormone, the slower the activity).

      Endocrine glands

      illustration


     

    Review Date: 5/20/2016

    Reviewed By: Todd Gersten, MD, Hematology/Oncology, Florida Cancer Specialists & Research Institute, Wellington, FL. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.

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